联合应用客观听力检测核黄疸新生儿的听觉通路损伤

目的:应用客观听力检测技术分析新生儿核黄疸后听觉通路损伤的临床听力学特点,评价感音神经性耳聋患儿的蜗性及蜗后病变特征。方法:选择1999-06/2003-12就诊的2岁以下新生儿核黄疸后伴听觉通路损伤患者32例(62耳)为核黄疸组;以大脑半球病变为主的疾病,如新生儿缺血缺氧性脑病恢复期、精神和运动发育迟缓等其它中枢性病变伴听觉通路损伤患儿34例(68耳)为中枢性病变组;并选取同年龄段听力正常小儿30例(54耳)为健康对照组。同时进行脑干听觉诱发电位、畸变产物耳声发射和镫骨肌声反射检测,对比不同组别间脑干听觉诱发电位波V阈值及畸变产物耳声发射各自的特点,同一组间不同脑干听觉诱发电位波V阈值耳畸变产物耳声发射的变化特征。各组间畸变产物耳声发射幅值的差异显著性选用方差分析,11个频点引出率等计数资料的差异显著性选用卡方检验。结果:各组患儿治疗过程中无脱落,均纳入结果分析。核黄疸组脑干诱发电位波V阈值>80nHLdB占81%,-80nHLdB占3%,-60nHLdB占16%;中枢性病变组分别为46%、28%、26%,分布比例较为均衡。核黄疸组脑干诱发电位波V阈值在-60nHLdB和>80nHLdB的F5频点后畸变产物耳声发射幅值差异无显著性(P>0.05),但低于中枢性病变组(P<0.05);核黄疸组和中枢性病变组听阈值在-60nHLdB的F5频点后畸变产物耳声发射检出率差异无显著性(χ2=0.72,P>0.05)。中枢性病变组听阈值在-60nHLdB和-80nHLdB听阈值F5频点后的畸变产物耳声发射幅值与对照组差异无显著性(P>0.05);-60nHLdB,-80nHLdB,>80nHLdB听阈值F5频点后的畸变产物耳声发射检出率与健康对照组相比差异无显著性(χ2=5.76,P>0.05)。结论:新生儿核黄疸后听觉通路损伤患者可联合应用客观听力检测进行评估,脑干听觉诱发电位检测和畸变产物耳声发射检测应成为临床上测试听力的两项基本无创客观手段。     

新生儿黄疸的脑干听觉诱发电位观察

新生儿黄疽又称新生儿高胆红素血症,是指新生儿时期由于胆红素代谢异常引起血中胆红素水平升高而出现的皮肤、巩膜及粘膜黄染的临床现象,严重者可发生胆红素脑病(核黄疽),听力障碍是其中枢神经系统受累的临床表现之一。 作者1996年对30例新生儿黄疽患儿进行了脑干听觉诱发电位(BAEP)的观察。报道如下:     

脑干听觉诱发电位在新生儿黄疸中的应用

新生儿黄疸是新生儿期常见的现象 ,近年来发现新生儿血清胆红素在较低水平时即可引起听力及神经系统功能损害 ,因此对于黄疸的患儿进行早期的听觉诱发电位检查具有重要的意义。我们通过对 5 6例高胆红素血症新生儿和正常组对照分析其脑干听觉诱发电位结果 ,发现 36例有异常 ,随着黄疸的消退 ,又渐恢复正常 ,现总结报告如下。1　资料与方法1.1　选择对象　 2 0 0 0年 1月至 2 0 0 2年 2月 ,我科收住的高胆红素血症的新生儿 (高胆组 ) 5 6例 ,其中男 32例 ,女 2 4例 ,胎龄 38～ 4 2周 ,体重 2 5 0 0～ 4 0 0 0 g ;血清胆红素水平在 2 10～ 395…     

高危新生儿脑干听觉诱发电位观察

目的：观察高危新生儿脑干诱发电位（BAEP）的变化,探索早期发现高危新生儿听力损害及脑干损害诊断方法。方法：用神经电位仪记录16例高危新生儿BAEP,并与正常新生儿作比较。结果：高危新生儿组BAEP的V波潜伏期（PL）,Ⅲ-Ⅴ、I-Ⅴ波间期（IPL）较对照组明显延长,差别有显著性。结论：高危新生儿易出现听力及脑干损害,BAEP对早期发现此损害,指导临床治疗有重要意义。     

脑干听觉诱发电位检测在新生儿胆红素脑病中的临床应用

【目的】观察胆红素脑病对新生儿外周及脑干听觉传导通路的影响。【方法】对40例胆红素脑病 的新生儿进行脑干听觉诱发电位(BAEP)检测,并以同龄儿正常值为对照。【结果】BAEP异常率95%,主要 表现在Ⅰ、Ⅲ、Ⅴ部分或全部消失,各波潜伏期(pl)延长,Ⅲ～Ⅴ或Ⅰ～Ⅲ波间期延长,Ⅴ波反应阈增高等。 【结论】胆红素脑病的新生儿外周及脑干听觉传导通路均有明显损害,BAEP对新生儿胆红素脑病的辅助诊断 及预后判断有重要价值。     

对新生儿听力普遍筛查模式的研究

目的探讨开展新生儿听力普遍筛查的模式,获得新生儿听力损失发病的初步资料.方法采用耳声发射和脑干诱发电位两步筛查法.新生儿出生3～4d畸变产物耳声发射(DPOAE)进行初次听力筛查,出生42d时再次用同样方法对所有接受过初筛的婴儿进行复筛,复筛未通过者脑干诱发电位(ABR)诊断,异常者在6个月时再次复查ABR,两次ABR未通过者为听力损失.所有数据采用SPSS统计软件进行统计分析.结果住院期间筛查新生儿3944例,一次通过3242例,通过率82.2%,42d再次复查3284例,通过3167例,通过率96.4%,未通过117例中37例(31.6%)初筛通过.复筛未通过者ABR检查72例,有"例3个月内ABR未通过,至6个月时再次复查ABR,有20例转为正常,最后确诊不同程度听力损失7例,听力损失发病率1.77‰,其中1例DPOAE初筛通过.结论在我国开展新生儿听力筛查是完全可行的,住院期间普遍初筛和出生42d普遍复筛有利降低筛查假阴性和假阳性,两次ABR确诊有利提高诊断准确性,是较适合的模式,值得探讨和推广.新生儿听力损失发病率与文献报道相似.     

137例早产儿听力检测

目的 探讨早产儿听力检测的临床意义。方法 对2003年5月～2005年9月在本院儿科和母婴同室住院的137例早产儿（其中无合并症早产儿67例、有合并症早产儿70例）和对照组足月新生儿2152例采用畸,变产物耳声发射（DPOAE）进行听力检测,早产儿组初检时间为出生后3—5d,复检时间为出生后42d和90d;对照组初检时间为出生后3～5d．发现听力异常者在出生后42d和90d进行复检。如3次检测均未通过者,进行脑干诱发电位（ABR）检测。结果 早产儿组听力障碍4例,异常率为2．92％,足月儿组听力障碍2例,异常率为0．09％,早产组与足月儿组比较,差异具有统计学意义（P〈0．05）;有合并症早产儿组异常率为5．71％,无合并症早产儿组异常率为0,有合并症组与见合并症组比较,差异具有统计学意义（P〈0．05）。结论 早产儿尤其是有合并症的早产儿是发生听力障碍的高危群体。     

宁波市听力筛查阳性婴幼儿客观听力检测

目的　探讨听性脑干反应（auditory brain stem response, ABR）和多频稳态诱发电位（auditory steady state response,ASSR）在听力筛查阳性婴幼儿听力诊断中的应用价值。方法　2005年8月至2007年10月期间宁波市新生儿畸变产物耳声发射（distortion product otoacoustic emission, DPOAE）听力筛查阳性者70例转诊至宁波市儿童听力诊断治疗中心进行听性脑干反应及多频稳态诱发电位测试,综合分析ABR及ASSR的测试结果。结果　70例婴幼儿（140耳）中ABR正常37耳, 轻度聋18耳, 中度聋21耳, 重度聋31耳和极重度聋33耳; 非极重度聋ASSR的2 kHz和4 kHz反应阈与ABR反应阈之间呈直线正相关关系（IP/I0.05）, 而ASSR的0.5 kHz和1 kHz反应阈与ABR反应阈之间无直线关系（IP/I0.05）。极重度聋0.5 kHz、1 kHz、2 kHz和4 kHz ASSR引出率分别为42.4%、57.6%、45.5%和48.5%,高于ABR的引出率21.2％（I/Isup2/sup=9.7408,IP/I=0.0450）。结论　听力筛查阳性婴幼儿的高载波频率ASSR与ABR存在良好的相关性,且ASSR具有频率特性及较高反应引出率而更适用于极重度聋婴幼儿残余听力的评估。     

脑干听觉诱发电位对4696例新生儿听力筛选的研究

目的探讨脑干听觉诱发电位应用丁新生儿听力筛选的可行性与新生儿听力损失的相关因素。方法采用脑干听觉诱发电位进行新生儿听力筛选,于出生后3～7d进行初筛,初筛未通过者于生后42d复筛,复筛未通过者于生后3个月再复查,仍未通过者确诊为听力损失。结果4696例新生儿进行筛查,初筛率95．7％。经初筛通过了4268例（90．9％）,初筛未通过428例（包括双侧或单侧朱通过）。其中394例进行了复筛,复筛率92．6％,复筛通过了326例（82．7％）,复筛未通过68例（17、3％）。其中66例于出生满3个门后再进行了复查,复查率97．1％,经复查54例通过了再复查,16例仍朱通过哲确诊为听力损失,占正常新生儿的0．34％。其中双侧均未通过5例,单侧未通过11例。结论新生儿听力筛查十分必要。脑干听觉诱发电位是一种快速、准确而行之有效的新生儿听力筛查疗法,可及早发现听力损失,以便进行早期干预;研究发现,听力障碍的发生,与听力障碍家旅史、耳毒性药物应用史、母孕病毒感染史、宫内窒息史、早产、婴儿患病史等有关,值得临床上重视。     

Bilateral sensorineural hearing loss due to mumps

Viral infections implicated in acute hearing loss include rubella, measles, mumps, herpes zoster, cytomegalovirus and influenza. Mumps' deafness is the best documented disorder. We describe a case where bilateral sensorineural hearing loss, following a severe mumps infection at the age of two, remained unnoticed until the age of four when the patient presented with delayed speech and language. This report emphasises the risk of permanent hearing loss as a complication of mumps infection and discusses strategies for early diagnosis and prevention.     

Imaging evaluation of sensorineural hearing loss

The imaging evaluation of patients with sensorineural hearing loss (SNHL) focuses on the acoustic pathways from the cochlea to the auditory cortex. Magnetic resonance imaging (MRI) is the modality of choice for most patients with SNHL, though computed tomography (CT) also plays an important role in the evaluation of bony changes and in patients for whom MRI is contraindicated. Conventional enhanced MRI is the most commonly used technique in this clinical setting. High-resolution fast spin-echo T2 MRI is an adjunctive technique that provides exquisite evaluation of the cerebellopontine angle (CPA), internal auditory canal (IAC), cranial nerves, and membranous labyrinth, and plays a significant role in the diagnosis and surgical evaluation of SNHL. Categories of lesions that cause SNHL include brain lesions involving central auditory pathways; neoplasms of the CPA and IAC, the most common being schwannoma; other neoplastic, congenital, and cystic masses of the CPA and IAC; congenital anomalies of the inner ear; intrinsic cochlear nerve defects, inflammatory processes of the inner ear; and temporal bone trauma.     

Hereditary non-syndromic sensorineural hearing loss: transforming silence to sound

Tremendous progress has been made in our understanding of the molecular basis of hearing and hearing loss. Through recent advances, we have begun to understand the fascinating biology of the auditory system and unveiled new molecular mechanisms of hearing impairment. Changes in the diagnostic impact of genetic testing have occurred, as well as exciting developments in therapeutic options. Molecular diagnosis, which is already a reality for several hearing-associated genes, will doubtlessly continue to increase in the near future, both in terms of the number of mutations tested and the spectrum of genes. Genetic analysis for hearing loss is mostly used for diagnosis and treatment, and relatively rarely for reproductive decisions, in contrast to other inherited disorders. Inherited hearing loss, however, is characterized by impressive genetic heterogeneity. An abundance of genes carry a large number of mutations, but specific mutations in a single gene may lead to syndromic or non-syndromic hearing loss. Some mutations predominate in individual ethnic groups. For clinical and laboratory diagnosticians, it is challenging to keep abreast of the unfolding discoveries. This review aims to provide the framework pertinent to diagnosticians and a practical approach to mutation analysis in the hearing impaired.     

老年突发性耳聋患者的预后分析

目的探讨老年突发性耳聋的临床特点及疗效预后。方法回顾性分析2008年1月至2015年12月扬州市第一人民医院耳鼻咽喉头颈外科住院,年龄在60岁以上(含60岁)的261例老年突发性耳聋患者的临床资料,了解其临床特征并分析不同因素(包括性别,侧别,初诊时间,不同听力曲线类型,是否伴发高血压,糖尿病等)对预后的影响。结果 261例老年突发性耳聋患者中,单侧耳聋240例(92.0%),双侧21例(8.0%);其中男性129例,女性132例;年龄60~84岁,平均(68.2±12.3)岁;发病至就诊时间0.5~62 d,中位时间4 d;患者听力曲线中全聋型115例(44.1%),中高频下降型63例(24.1%),平坦型56例(21.5%),低中频下降型27例(10.3%)。治疗后的总有效率分别为54.8%(63/115)、44.4%(28/63)、71.4%(40/56)和81.5%(22/27);261例患者经过规范的药物治疗,痊愈45例,好转106例,未愈110例,治疗总有效率为57.9%(151/261)。经过统计学分析,不同患者听力曲线类型,侧别,初诊时间总有效率有统计学差异(P<0.05)。来自城市与农村患者经规范治疗后总有效率比较差异有统计学意义(P<0.05)。结论老年突发性耳聋一般听力损失重,但及时治疗,患者仍有恢复的可能。单侧老年突聋患者较双侧预后好;听力曲线以全聋型和中高频下降型居多,预后以低中频下降型最好。患者初诊时间越短,疗效越好;来自城市的老年突聋患者预后较好。     

自身免疫性感音神经性聋发病机制及临床分型

自身免疫性感音神经性聋不仅损伤耳蜗，而且损伤蜗后，本文重点介绍发病机制、组织病理学改变、临床分型和鉴别诊断.     

Comparison of immune assays for the detection of anti-HSP70 antibodies in patients with idiopathic sensorineural hearing loss

BACKGROUND: Detection of anti-heat-shock protein 70 (HSP70) IgG response by Western blot (WB) is of clinical utility in a subset of patients with idiopathic sensorineural hearing loss (SNHL) due to autoimmunity. METHODS: To validate an immune assay for the detection of anti-HSP70 antibody responses in the clinical laboratory, we employed a commercial anti-human HSP70 IgG/A/M ELISA and developed an anti-HSP70 IgG WB test. Using sera from 81 patients with idiopathic SNHL and 100 healthy controls, we assessed each assay performance with results from another diagnostic laboratory that utilizes a WB test. RESULTS: Our results showed a significant lack of agreement between either WB assay and the anti-human HSP70 IgG/A/M ELISA for antibody-positive samples. Comparison of WB assays revealed a significant level of agreement (89.7%) for all samples tested. CONCLUSIONS: Our data suggest that the antigenic targets in WB and ELISA immunoassays differ and demonstrate that the anti-HSP70 IgG WB test is reproducible within and between clinical laboratories. Thus, in the absence of disease-specific markers, the anti-HSP70 IgG WB assay could be of use to detect patients with idiopathic SNHL who might benefit from steroid treatment.     

突发性耳聋病人焦虑状况分析及护理对策

目的分析突发性耳聋住院病人焦虑状况及其影响因素,探讨如何采取有效的心理护理对策,减轻病人的焦虑状况,提高治疗效果。方法应用焦虑自评量表(SAS)及自制调查问卷分析评估138例突发性耳聋住院病人焦虑状况及其相关因素。结果122例出现不同程度的焦虑,占88.4%;焦虑发生及程度受听力恢复程度、耳鸣、疾病知识缺乏及经济负担能力等因素影响;以20~40岁和40~60岁这两年龄段高发,分别为46.7%和34.4%。结论对突发性耳聋病人加强心理护理、健康教育及改善住院环境,可减轻病人的焦虑情绪,促进恢复。     

Treatment of peripheral sensorineural hearing loss: gene therapy

Noise, chemicals and genetic defects are all common causes of irreversible hearing loss, which at present have no cure. Gene therapy may soon be utilized in both the protection and the treatment of these exogenous and endogenous sources of hearing loss. Gene therapy technology is rapidly developing and the inner ear is a particularly feasible model for gene therapy. This review outlines our current understanding of the mechanisms behind deafness and prospects for treatment, discusses the inner ear model in detail and reviews the efforts that have been made in inner ear gene therapy. Finally, the proposed next steps will be discussed. The viral mediated delivery of neurotrophins and antioxidants offers imminent promise in preventing and treating exogenous hearing loss and improving cochlear implant therapy.